Assessing the role of immuno-proteasomes in a mouse model of familial ALS

Krishna Puttaparthi, Luc Van Kaer, Jeffrey L. Elliott

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

The accumulation of protein aggregates is thought to be an important component in the pathogenesis of mutant SOD1-induced disease. Mutant SOD1 aggregates appear to be cleared by proteasomes, at least in vitro, suggesting a potentially important role for proteasome degradation pathways in vivo. G93A SOD1 transgenic mice show an increase in proteasome activity and induction of immuno-proteasome subunits within spinal cord as they develop neurological symptoms. To determine what role immuno-proteasomes may have in mutant SOD1-induced disease, we crossed G93A SOD1 transgenic mice with LMP2-/- mice to obtain G93A SOD1 mice lacking the LMP2 immuno-proteasome subunit. G93A SOD1/LMP2-/- mice show significant reductions in proteasome function within spinal cord compared to G93A SOD1 mice. However, G93A SOD1/LMP2-/- mice show no change in motor function decline, or survival compared to G93A SOD1 mice. These results indicate that the loss of immuno-proteasome function in vivo does not significantly alter mutant SOD1-induced disease.

Original languageEnglish (US)
Pages (from-to)53-58
Number of pages6
JournalExperimental Neurology
Volume206
Issue number1
DOIs
StatePublished - Jul 2007

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Proteasome Endopeptidase Complex
Transgenic Mice
Spinal Cord

Keywords

  • Aggregation
  • ALS
  • Astrocyte
  • Microglia
  • Motor neuron
  • Spinal cord

ASJC Scopus subject areas

  • Neurology
  • Neuroscience(all)

Cite this

Assessing the role of immuno-proteasomes in a mouse model of familial ALS. / Puttaparthi, Krishna; Van Kaer, Luc; Elliott, Jeffrey L.

In: Experimental Neurology, Vol. 206, No. 1, 07.2007, p. 53-58.

Research output: Contribution to journalArticle

Puttaparthi, Krishna ; Van Kaer, Luc ; Elliott, Jeffrey L. / Assessing the role of immuno-proteasomes in a mouse model of familial ALS. In: Experimental Neurology. 2007 ; Vol. 206, No. 1. pp. 53-58.
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